Pituitary Tumors

A Comprehensive and Interdisciplinary Approach

1st Edition - April 11, 2021
Editors: Jürgen Honegger, Martin Reincke, Stephan Petersenn
Language: English
Hardback ISBN:
9 7 8 - 0 - 1 2 - 8 1 9 9 4 9 - 7
eBook ISBN:
9 7 8 - 0 - 1 2 - 8 1 9 9 5 0 - 3

Pituitary Tumors: A Comprehensive and Interdisciplinary Approach provides the latest information on preclinical issues, diagnostic procedures, treatment options and post-t… Read more

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Pituitary Tumors: A Comprehensive and Interdisciplinary Approach provides the latest information on preclinical issues, diagnostic procedures, treatment options and post-treatment care for patients with pituitary tumors. The book includes basic and advanced knowledge for a broad audience, including physicians, endocrinologists, neurosurgeons, neuro-radiologists, neuro-ophthalmologists, neuro-pathologists, oncologists, radiotherapists and researchers who are investigating pituitary tumors. Readers will find the latest research surrounding progress on uncoding the molecular mechanisms involved in tumor genesis. In addition, standard treatment modalities, including surgery, medical treatment and radiosurgery are explored.

Cover image
Title page
Table of Contents
Copyright
Contributors
About the editors
Foreword
Preface
Section I: Preclinical essentials
Chapter 1: Physiology of pituitary hormones
Abstract
Introduction
Hypothalamic-pituitary-adrenal axis
Gonadotropic axis
Somatotropic axis
Thyrotropic axis
Lactotropic axis
Hormones of the posterior pituitary
Key points
Chapter 2: Principles of laboratory investigation for pituitary hormones
Abstract
Preanalytical aspects
Analytical aspects
Postanalytical aspects (interpretation)
Key points
Chapter 3: Pathophysiology and genetics in pituitary tumors
Abstract
Introduction
Pathophysiology
Hypothalamic regulation
Peripheral negative feedback
Genetics of pituitary tumorigenesis
Pituitary tumors in syndromes
Sporadic pituitary tumors
Conclusion
Key points
Chapter 4: Pathophysiology and genetics in craniopharyngioma
Abstract
Acknowledgments
Introduction
Genetic alterations in craniopharyngioma
Preclinical models of craniopharyngioma
Cell culture models
Models of PCP
Role of the clusters in ACP
Senescence
The molecular relationship between ACP and the developing tooth
Developmental pathways activated in craniopharyngioma
The MAPK pathway
The sonic hedgehog pathway
The TGFβ and BMP pathways
The Hippo pathway
Expression of inflammatory factors in ACP
Angiogenesis
Tumor recurrence
Summary and future directions
Key points
Chapter 5: Anatomy of the pituitary region
Abstract
Introduction
Nasal cavities
Paranasal sinuses
Skull base
Hypothalamus
Pituitary gland
Diaphragma sellae
Cavernous sinus
Internal carotid artery and its branches
Cranial nerves I–VI
Key points
Section II: Neuroradiological and ophthalmological diagnosis in pituitary tumors
Chapter 6: Morphological imaging including imaging anatomy
Abstract
Introduction
Magnetic resonance imaging
MR imaging of adenoma
Computed tomography (CT)
Digital subtraction angiography
Key points
Chapter 7: Functional imaging
Abstract
Introduction
Somatotroph adenomas
Corticotroph adenomas
Lactotroph adenomas
Conclusions
Key points
Chapter 8: Neuroophthalmological diagnosis
Abstract
Introduction
Neuroophthalmological examination techniques
History
Neuroophthalmological routine examination
Visual field testing
Morphological studies
Electrophysiological studies
Typical neuroophthalmological signs of pituitary tumors
Neuroophthalmological symptoms of chiasmal visual loss
Key points
Section III: Endocrinological diagnosis and replacement therapy for hypopituitarism and diabetes insipidus in pituitary tumors
Chapter 9: Endocrinological diagnosis and replacement therapy for hypopituitarism
Abstract
Introduction
Historical prospective
Clinical symptoms and signs
Endocrine evaluation and replacement therapies
Hormone-hormone interaction
Limitation with current replacement strategy
Hypopituitarism during pregnancy
Summary
Key points
Chapter 10: Endocrinological diagnosis and replacement therapy for diabetes insipidus
Abstract
Prevalence and definition of the polyuria polydipsia syndrome
Causes of central diabetes insipidus
Diagnosis
Replacement therapy
Key points
Chapter 11: The role of nurses in supporting self-management for patients with hypopituitarism
Abstract
Introduction
Hormone replacement therapy in hypopituitarism
The remit of nurses in the care of patients with hypopituitarism
Improving adherence to pituitary replacement therapy
Patient education approaches in supporting self-management
Conclusion
Key points
Section IV: Endocrinological diagnosis and medical treatment in functioning pituitary adenomas
Chapter 12: Prolactinomas: Diagnosis and medical treatment
Abstract
Introduction
Epidemiology
Clinical presentation
Diagnosis
Other etiologies of hyperprolactinemia
Medical treatment
Resistant prolactinomas
Adverse effects of dopamine agonists
Children and adolescents
Giant prolactinomas
Cystic prolactinomas
Pasireotide
Temozolomide
Conclusions
Key points
Chapter 13: Endocrinological diagnosis in acromegaly
Abstract
Physiology of the hypothalamic-pituitary-somatotropic axis
Epidemiology
Etiology
Signs and symptoms
Diagnosis
Burden of disease and health care cost
Summary
Key points
Chapter 14: Medical treatment in acromegaly
Abstract
Introduction
Therapeutic goals
Treatment options
First-generation SRLs
Second-generation SRLs (pasireotide)
Dopamine agonists (DA)
GH receptor antagonist (pegvisomant)
Combination therapies
Perspectives
Conclusions
Key points
Chapter 15: The diagnosis of Cushing's disease
Abstract
Clinical diagnosis
Biochemical diagnosis
Bilateral inferior petrosal sinus sampling (BIPSS)
Imaging
Conclusions
Key points
Chapter 16: Medical management of Cushing's disease
Abstract
Acknowledgments
Introduction
Adrenal-directed therapy
Pituitary-directed therapy
Emerging treatment
Medical management of Cushing's disease in pregnancy
Monitoring
Combination therapy
Severe hypercortisolemia and psychosis
Key points
Chapter 17: Endocrinological diagnosis and treatment of TSH-secreting pituitary adenomas
Abstract
Introduction
Epidemiology
Pathophysiology and etiologic forms
Clinical features and diagnosis
Treatment
Conclusions
Key points
Section V: Surgical treatment of pituitary tumors
Chapter 18: Microscopic transsphenoidal surgery
Abstract
General aspects
Patient positioning
Surgical technique
The perinasal-paraseptal route
The sublabial incision
The direct endonasal approach
Sphenoidotomy and exposure of the sellar floor
Opening the sella and tumor resection
Reconstruction of sellar floor and closure
Postoperative management
Key points
Chapter 19: Endoscopic transsphenoidal surgery: Including a brief history of transsphenoidal surgery
Abstract
Historical perspective of (endoscopic) transsphenoidal surgery
Invention of endoscopy
Evolution of endoscopic transsphenoidal surgery
Surgical aspects
Endoscopic endonasal surgery for pituitary tumors
Surgical pearls and pitfalls
Endoscopic surgery for recurrent adenomas
Comparison of the surgical results using the endoscopic and microsurgical technique
Further developments
Key points
Chapter 20: Risks of transsphenoidal surgery
Abstract
Introduction
Patient selection, approach selection, and surgeon experience
Surgical setup and technology adjuncts for endoscopic pituitary surgery
Sellar exposure and carotid artery localization
Postoperative gland dysfunction, cranial nerve neuropathies, and bleeding
Skull base reconstruction
Postoperative surveillance
Minor complications
Key points
Chapter 21: Ophthalmologic outcome of transsphenoidal surgery
Abstract
Introduction
Recovery of visual and oculomotor function
Predictors of visual outcome
Follow-up recommendations
Recommended ophthalmological tests
Key points
Chapter 22: Endocrinological outcome of transsphenoidal surgery in pituitary adenomas
Abstract
Introduction
Cushing's disease
Acromegaly
Prolactinoma
Thyrotropin-producing adenoma
Nonfunctioning pituitary (macro-)adenoma
Large or giant pituitary adenoma
Mixed cohorts
Transsphenoidal pituitary surgery in special populations
Endoscopic versus microscopic transsphenoidal surgery for pituitary adenomas
Experience with transsphenoidal surgery
Key points
Chapter 23: Extended transsphenoidal surgery
Abstract
Conflict of interest
Introduction
Surgical implications of giant pituitary adenomas
Indications of the extended endoscopic endonasal approach in the treatment of pituitary adenomas
Preoperative evaluation
Anatomical correlation and applied anatomy
The extended endoscopic endonasal approach: Surgical tenets and description of the technique
Limitations
Complications associated with the technique
How to avoid complications
Key points
Chapter 24: Transcranial surgery
Abstract
Historical perspectives
Indications for transcranial surgery
Preoperative preparation
Operative techniques for transcranial approaches
Conclusion
Key points
Chapter 25: Video presentations of pituitary surgery
Abstract
Section VI: Histo-pathological classification of pituitary tumors
Chapter 26: Pathology of pituitary adenoma
Abstract
Definition
Epidemiology
Etiology and (molecular) pathogenesis
Classification
Aggressive adenomas
Pituitary carcinomas
Classification of Trouillas et al. (2015)
PitNET (pituitary neuroendocrine tumor) instead of pituitary adenoma
Key points
Chapter 27: Pathology of nonadenomatous pituitary tumors and tumor-like lesions
Abstract
Craniopharyngiomas
Tumors of the neurohypophysis: TTF-1-positive spindle cell tumors
Other tumors of the sellar region
Metastases in the pituitary
Tumor-like lesions
Key points
Section VII: Perioperative and postoperative management in patients with pituitary adenomas
Chapter 28: Perioperative neurosurgical management
Abstract
Introduction
Preoperative workup
Informed consent
Anesthesiological considerations for pituitary surgery
Perioperative pain management
Perioperative management of anticoagulation
Postoperative surgical order
Postoperative level of care
Perioperative glucocorticoid management
Secondary hypothyroidism
Perioperative management of electrolyte and fluid balance
Management of CSF leakage
Postoperative imaging
Otorhinolaryngological management after transsphenoidal surgery
Postoperative recommendations after transsphenoidal surgery
Checklist for discharge
Key points
Chapter 29: Perioperative endocrinological management in patients with pituitary adenomas
Abstract
Introduction
Central adrenal insufficiency
Water balance disorders
Assessment of remission in patients with hormone-secreting pituitary adenomas
Conclusions
Key points
Chapter 30: Perioperative and postoperative nursing care
Abstract
Institutional impact to care rendered
Preoperative nursing care
Postoperative nursing considerations
Monitoring for endocrinologic dysfunction
Preparing for hospital discharge and follow up coordination
Key points
Chapter 31: Postoperative endocrinological follow-up
Abstract
Introduction
Assessment of anterior pituitary function after surgery (see Table 1)
Assessment of cure after surgery
Concluding remarks
Key points
Chapter 32: Pituitary magnetic resonance imaging use in the posttreatment follow-up of secreting pituitary adenomas
Abstract
Introduction
Pituitary magnetic resonance imaging
2017 WHO classification of pituitary tumors
Postsurgical surveillance of pituitary tumors
Surveillance during medical therapy
Surveillance after radiotherapy/radiosurgery
Acromegaly
Cushing's disease
Prolactin-secreting tumors
Key points
Section VIII: Radiotherapy for pituitary adenomas
Chapter 33: Fractionated radiotherapy for pituitary adenomas
Abstract
Introduction
Treatment indication
Treatment plan and application
Follow-up
Treatment outcomes
Radiotherapy-related toxicities
Hypofractionated stereotactic radiotherapy
Key points
Chapter 34: Pituitary adenomas: Radiosurgery
Abstract
Background
Efficacy on hormone hypersecretion
Control of tumor growth
Side effects
Conclusions
Key points
Section IX: Aggressive pituitary adenomas and carcinomas
Chapter 35: Management of aggressive pituitary tumors
Abstract
Introduction
Definition of an aggressive pituitary tumor
Invasiveness
Tumor growth
Optimal standard medical treatment
Diagnosis and follow-up of an aggressive pituitary tumor
Pituitary carcinomas
Role and limits of the neurosurgeon
Potential factors implicated in aggressiveness
Conclusion
Key points
Chapter 36: Medical treatment of aggressive pituitary tumors
Abstract
Introduction
Chemotherapy
Off-label use of drugs licensed for other pituitary tumor entities
Experimental therapies
Conclusions
Key points
Section X: Diagnosis, treatment and outcome in nonfunctioning pituitary tumors and lesions
Chapter 37: Clinically nonfunctioning pituitary tumors
Abstract
Introduction
Epidemiology
Classification of clinically nonfunctioning pituitary tumors
Clinical presentation
Diagnosis
Treatment and outcomes
Conclusions
Key points
Chapter 38: Childhood-onset craniopharyngiomas
Abstract
Acknowledgments
Introduction
Epidemiology
Pathology
Tumor location
Presenting clinical manifestations
Imaging studies
Treatment strategies
Risk factors and treatment for recurrences
Long-term outcome and sequelae
Questions and perspectives
Key points
Chapter 39: Adult craniopharyngiomas
Abstract
Introduction
Epidemiology
Pathogenetic background
Histopathology
Clinical presentation
Diagnostic workup
Therapy
Prognosis
Quality of life
Key points
Chapter 40: Rathke's cleft cysts and arachnoid cysts
Abstract
Introduction
Natural history
Diagnosis and management
Conclusion
Key points
Conflicts of interest
Disclosures
Chapter 41: Perisellar solid tumors
Abstract
Introduction
Optic pathway gliomas
Hypothalamic hamartomas
Intracranial germ cell tumors
Tumors of the posterior pituitary
Gangliocytomas and mixed gangliocytoma-pituitary adenomas
Perisellar meningiomas
Clivus chordomas
Petroclival chondrosarcomas
Metastatic pituitary tumors
Key points
Chapter 42: Inflammatory pituitary lesions
Abstract
Introduction
Primary hypophysitis
Secondary hypophysitis
Systemic, noninfectious granulomatous diseases
Sellar and parasellar diseases causing hypophysitis
Infectious diseases causing hypophysitis
Key points
Chapter 43: Vascular lesions and aneurysms
Abstract
Introduction
Cavernous malformations of the optic pathways
Cavernous sinus hemangiomas
Perisellar aneurysms
Coexistence of intracranial aneurysm and pituitary adenoma
Abnormal intrasellar arteries
Key points
Section XI: Diagnosis and treatment of co-morbidities in pituitary tumors
Chapter 44: Psychiatric disorders
Abstract
Introduction
Hormone excess syndromes and psychiatric disorders
Acromegaly
Cushing's disease
Prolactinomas
Rare diseases of the hypothalamus/pituitary gland and psychiatric disorders
Diagnosing and treating psychiatric disorders in pituitary tumors
Conclusion
Key points
Chapter 45: Metabolic disorders
Abstract
Introduction
Growth hormone
ACTH/cortisol
Prolactinomas
Hypopituitarism
Key points
Chapter 46: Osteoporosis and arthropathy in functioning pituitary tumors
Abstract
Disclosure statement
Introduction
Part A Acromegaly
Part B Cushing's disease
Part C Prolactinoma
Part D TSH-producing adenoma
Conclusion
Key points
Section XII: Special issues
Chapter 47: Pituitary incidentaloma
Abstract
Definition
Epidemiology
Etiology
Clinical features and differential diagnosis
Differential diagnosis
Natural history of pituitary incidentalomas
Evaluation
Treatment
Pituitary incidentalomas in special clinical situations
Follow-up
Key points
Chapter 48: Pituitary apoplexy
Abstract
Definition
The medical history of PA
Epidemiology of PA
Pathophysiology and risk factors of PA
Clinical presentation of PA
Imaging diagnostics
Laboratory findings in PA
Treatment of PA
Long-term endocrinological outcome and follow-up recommendations
Key points
Chapter 49: Management of pituitary tumors in pregnancy
Abstract
Introduction
Prolactinomas
Acromegaly
TSH secreting pituitary adenoma during pregnancy
Clinically nonfunctioning pituitary adenoma
Conclusions
Key points
Chapter 50: Quality of life in pituitary tumors
Abstract
Introduction
Quality of life in patients with adult growth hormone deficiency (AGHD)
Quality of life in patients with acromegaly
QoL in Cushing's syndrome
QoL in hyperprolactinemia/prolactinomas
QoL in NFPA
Examples of patient perceptions on QoL
Strategies for patients to improve QoL and pituitary disease perception
Conclusions
Key points
Chapter 51: Multidisciplinary team perspective: A model of care for patients with pituitary tumors
Abstract
Introduction
Goals and benefits of pituitary multidisciplinary teams
The structure of the pituitary multidisciplinary team
Proposed model of function of pituitary multidisciplinary team
Multidisciplinary team model as part of a Pituitary Tumor Center of Excellence
Conclusions
Key points
Index

Jürgen Honegger

Jürgen Honegger is Professor of Neurosurgery and Vice-Chairman of the Department of Neurosurgery at the Eberhard-Karls University in Tübingen, Germany. He worked as a resident in the ophthalmological department in Tübingen (1986) and as a resident (1986-1989), senior resident (1991-1994), and consulting neurosurgeon (1994-1997) in neurosurgery at the University of Erlangen-Nürnberg, where he was trained in pituitary surgery by Professor Rudolf Fahlbusch. He spent 1990 at St. Bartholomew’s hospital in London, UK, as a Clinical and Research Fellow to Professors Michael Besser, John Wass, and Ashley Grossman. From 1998 to 2004, he was consultant at the Department of Neurosurgery, University of Fribourg. Since 2005, he has been Vice-Chairman at the Department of Neurosurgery, University of Tübingen, where his main neurosurgical specialities are pituitary surgery, vascular surgery, and epilepsy surgery. Jürgen Honegger was licensed as neurosurgeon in 1987. He was appointed Associate Professor (with a thesis on “Biochemical characterization and surgical treatment of craniopharyngiomas”) in 1997 and Professor in Neurosurgery in 2006. His main clinical and scientific area of activity is in pituitary tumors. In Tübingen, he has built up a Pituitary Surgery Unit that today ranks among the largest units in Germany. Prof. Honegger performs more than 150 operations for pituitary tumors per year. He has organized and hosted pituitary surgery training courses of the German Society of Neurosurgery. He has directed several national studies and surveys on pituitary disorders. Since 2014, he has been the head of the Pituitary Tumor Working Group of the German Society of Endocrinology, which holds the patronage for the German Acromegaly Registry, the German Craniopharyngioma Registry for Adults, and the German Pituitary Tumor Registry.

Affiliations and expertise

Department of Neurosurgery, University of Tübingen, Tübingen, Germany

Martin Reincke

Martin Reincke is Professor of Endocrinology and Chair of Medical Department IV of the Ludwig-Maximilians University in Munich, a leading institution in German academic medicine. His research specialties include endocrine hypertension, mineralocorticoid and glucocorticoid action, and stress research. Professor Reincke is a clinician-scientist at heart. He heads a research team that specifically explores the prevalence and relevance of glucocorticoid and mineralocorticoid excess on the epidemiological, clinical, genetic, and molecular levels. He is co-founder of the European Network for the Study of Adrenal Tumors (ENSAT), established in 2000. In 2009, he founded the German Primary Aldosteronism Registry, and in 2012, the German Cushing Registry. Professor Reincke is a member of many national and international societies, including the Endocrine Society and the European Society of Endocrinology. He has served on the Executive Board of the German Endocrine Society and European Society of Endocrinology, is on the editorial boards of several international journals, and has served as Teaching Dean of the Faculty from 2006 to 2012 and as president of the German Endocrine Society (2014–2017). Prof. Reincke won the prestigious European Research Council Advanced Grant Award (2.5 million €) in 2016. He is currently President-Elect of the European Society of Endocrinology. His publications include more than 490 MEDLINE-listed manuscripts, which have been cited >12,500 times, and his h-index is 61 (ISI Web of Science).

Affiliations and expertise

Chairman of Medicine, Department of Medicine IV, University Hospital Munich, Ludwig-Maximilians-University Munich, Germany

Stephan Petersenn

Stephan Petersenn, MD, ENDOC Center for Endocrine Tumors, Hamburg, Germany. Prof. Stephan Petersenn studied medicine at the University of Kiel, Germany (1984–1987 and 1988–1990) and the University of Vienna, Austria (1987–1988). He is qualified in Internal Medicine (1999), Diabetology (1999), Endocrinology (2000), and Andrology (2007). Previously, he worked in an internship (1991–1992) in the Department of Medicine, University of Kiel, as a Research Fellow (1992–1994) to Professor S Melmed at Cedars-Sinai Medical Center, Los Angeles, USA, as a Resident and Fellow (1995–2000) in the Department of Medicine, University of Hamburg, and as a Senior Physician (2001–2009) in the Division of Endocrinology, University of Duisburg-Essen. In 2009, he founded the ENDOC Center for Endocrine Tumors in Hamburg, while remaining a member of the faculty of the University of Duisburg-Essen. In 2003, he became Associate Professor, and in 2008 Professor of Medicine, at the University of Duisburg-Essen. His research is focused on the diagnosis and treatment of pituitary and adrenal tumors, as well as neuroendocrine tumors. Furthermore, his clinical and basic studies aim for a better understanding of the physiology and pathophysiology of somatostatin receptors. Throughout his career, Prof Petersenn has published more than 160 articles in peer-reviewed journals of high reputation, and he has presented at many international conferences. He is a member of several societies, including the Endocrine society, the European Society of Endocrinology, and the International Pituitary Society.

Affiliations and expertise

ENDOC Center for Endocrine Tumors, Hamburg, Germany

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Pituitary Tumors

A Comprehensive and Interdisciplinary Approach

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Innovate. Sustain. Transform.

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Jürgen Honegger

Martin Reincke

Stephan Petersenn